B C Frykholm

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Acute intermittent porphyria (AIP) is a primary disorder of haem biosynthesis that is chemically characterised by raised urinary porphobilinogen (PBG). A defect in the biochemical pathway at the step of PBG conversion to uroporphyrinogen has been shown to be a result of a partial deficiency of the enzyme uroporphyrinogen I synthetase (uro I syn). The(More)
A thirty-three-year-old female with acute intermittent porphyria (A.I.P.) was having regular attacks of the disease with her menstrual periods. During several of these attacks she received intravenous haematin, which was followed by chemical and clinical remissions. Hormones failed to prevent the regular attacks, which were completely prevented by 200 mg of(More)
Two recognized screening procedures for rapid evaluation of urinary porphobilinogen (PBG) concentrations are compared with quantitative PBG determinations (expressed as mg/24 hours and as a concentration in urine, mg/liter). One hundred ninety-one 24-hour urine specimens from 74 patients with suspected or documented acute type porphyria are included in this(More)
Acute intermittent porphyria (AIP) is a disorder of porphyrin metabolism in which the basic defect is a partial deficiency of uroporphyrinogen I synthase. The clinical disorder is more common in women, and some experience acute attacks before menstrual periods. Oral contraceptives have prevented menstrual-associated attacks in some cases, but exogenous(More)